La Revue de médecine interne
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In Fabry disease, deficiency of α-galactosidase A results in the accumulation of glycosphingolipids in body fluids and tissues including corneas, blood vessels, kidneys and also structures of the central and peripheral nervous system. Many patients show cardiovascular and cerebrovascular dysfunction. Cerebrovascular dysfunction is particularly associated with a high risk of strokes and of mortality even at a young age. ⋯ Sympathetic dysfunction explains the hypohidrosis and a subsequent poor exercise and heat tolerance. Enzyme replacement therapy (ERT) with recombinant human α-galactosidase A is available. We could demonstrate improvement of small fiber neuropathy and neuropathic pain after 18-23 months of ERT, which probably resulted from glycosphingolipid clearing from perineurial cells, axons and Schwann cells or from blood vessels supplying the nerves.
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Increased production of TNFα by alveolar macrophages and involvement of TNFα in granuloma formation suggest that this cytokine is involved in the pathophysiology of sarcoidosis. The three available TNFα blocking agents have been tested in sarcoidosis refractory to corticosteroids or immunosuppressive drugs. Data are available from isolated case reports or limited series of patients treated in open label trials with favourable issue with anti-TNFα monoclonal antibodies. ⋯ A literature review identified 28 cases, including 16 with etanercept, eight with infliximab and four with adalimumab. Although these cases were mainly reported with etanercept, paradoxical sarcoidosis has been reported with the three available anti-TNFα agents, suggesting a class effect. Changes in the cytokine balance may be involved in these cases of induced sarcoidosis, which must be known by the clinician.
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In France, the analysis of the prescriptions by a pharmacist in hospital is mandatory since 1991. However, for various reasons, this activity remained poorly developed and little research has been performed. Consequently, this activity suffers of a lack of visibility to hospital decision-makers and others health care professionals. The aim of this paper is to describe drugs related problems identified by pharmacist prescriptions analysis on a large number of orders in a large teaching hospital. This was done in order to highlight recurrent and preventable problems. ⋯ These results should be used to reexamine hospital drug prescription policy. They prompt health care professionals to be aware about new medications errors potentially related to computerized prescription order entry. Finally, they invite to modify initial and continuous education programs of health care professionals.